SUE-HONG WANG2026-10-162026-10-162008-09-01Pure ID: 14910712Pure UUID: 7a99a10e-bf1d-4265-b246-1cc26fdb2a9a10.1016/j.nbd.2008.05.014https://www.scopus.com/pages/publications/49349107825https://scholars.csmu.edu.tw/handle/123456789/33996Currently, no curative treatment is available for spinal muscular atrophy (SMA). Since the degeneration of spinal motor neurons in SMA is mediated by apoptosis, over-expression of an anti-apoptotic factor, Bcl-xL, may benefit SMA. Here, we crossed a mouse model of SMA with Bcl-xL transgenic mice to create SMA/Bcl-xL mice. The Bcl-xL expression in the spinal neurons of SMA/Bcl-xL mice was nearly double that in SMA mice. SMA/Bcl-xL mice showed preserved motor function, normalized electrophysiological tests, diminished muscle atrophy, and less motor neuron degeneration. In addition, the life span of SMA/Bcl-xL mice was 1.5 times longer than that of SMA mice. Therefore, over-expression of Bcl-xL has a potential for amelioration of SMA, and Bcl-xL may be another attractive therapeutic target other than survival motor neuron (SMN) protein for use in future drug screening for SMA.7enjournal article